Ph. Roche et al., SKULL BASE CHONDROSARCOMA - REPORT OF A S PHENOTEMPORAL LOCALIZATION AND REVIEW OF THE LITERATURE, Neuro-chirurgie, 41(5), 1995, pp. 353-358
A review of the 180 published cases emphasizes the rarity of this tumo
ur originating of the skull base synchondrosis and occuring in young p
atients. MRI forms the basis of the topographic work-up but does not f
ormally distinguish it from chordoma. Diagnosis is confirmed by histol
ogy which establishes a prognostic scale. Immunohistochemistry elimina
tes chondroid chordoma by demonstrating a mesenchymatous phenotype. On
ly enlarged tumour removal can prolong survival even though improvemen
ts have been made in high energy radiation therapy and radiosurgery. C
ases of recurrence or rare metastasis are probably due to incomplete e
xeresis and in most cases linked to high grade histology tumours.